Hamuryudan V, Yurdakul S, Moral F, Numan F, Tüzün H, Tüzüner N, Mat C, Tüzün Y, Ozyazgan Y, Yazïci H
Department of Rheumatology, Cerrahpaşa Medical Faculty, University of Istanbul, Turkey.
Br J Rheumatol. 1994 Jan;33(1):48-51. doi: 10.1093/rheumatology/33.1.48.
Pulmonary arterial involvement is an important complication of Behçet's syndrome (BS). Among 2179 patients with BS, 24 (1.1%) were diagnosed as having pulmonary arterial aneurysms (PAAs). Haemoptysis was the presenting symptom in all but one. All were male. The mean age at the time of the diagnosis of PAA was 30 +/- 11 S.D. yr (range 17-59 yr). Their mean disease duration was 5 +/- 4 yr (range 3 months-16 yr). There was a high prevalence of thrombophlebitis (21/24, 88%). Histopathological examination showed pulmonary vasculitis involving all layers of pulmonary arteries and veins. Twelve patients (50%) died after a mean of 9.5 +/- 11 S.D. months (range 1-36 months) after the onset of haemoptysis. The mean duration of follow-up of the remaining 12 patients was 25.5 +/- 24 S.D. months (range 1-78 months). The treatment consisted mainly of pulsed or oral cyclophosphamide alone or with prednisolone. As is true with other severe manifestations of Behçet's syndrome, PAAs are more common among males. They are associated with a prevalence of thrombophlebitis and there is high mortality despite treatment.
肺动脉受累是白塞病(BS)的一种重要并发症。在2179例白塞病患者中,24例(1.1%)被诊断为患有肺动脉瘤(PAA)。除1例以外,咯血是所有患者的首发症状。所有患者均为男性。诊断PAA时的平均年龄为30±11标准差岁(范围17 - 59岁)。他们的平均病程为5±4年(范围3个月 - 16年)。血栓性静脉炎的患病率很高(21/24,88%)。组织病理学检查显示肺血管炎累及肺动脉和肺静脉的所有层。12例患者(50%)在咯血发作后平均9.5±11标准差个月(范围1 - 36个月)死亡。其余12例患者的平均随访时间为25.5±24标准差个月(范围1 - 78个月)。治疗主要包括单独使用脉冲或口服环磷酰胺或与泼尼松龙联合使用。与白塞病的其他严重表现一样,PAA在男性中更为常见。它们与血栓性静脉炎的患病率相关,并且尽管进行了治疗,但死亡率仍然很高。